Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000793049 | SCV000932384 | pathogenic | Neurofibromatosis, type 1 | 2018-08-18 | criteria provided, single submitter | clinical testing | Loss-of-function variants in NF1 are known to be pathogenic (PMID: 10712197, 23913538). For these reasons, this variant has been classified as Pathogenic. This variant has not been reported in the literature in individuals with NF1-related disease. This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Leu113Phefs*14) in the NF1 gene. It is expected to result in an absent or disrupted protein product. |
Ambry Genetics | RCV002442618 | SCV001181611 | pathogenic | Hereditary cancer-predisposing syndrome; Cardiovascular phenotype | 2018-06-29 | criteria provided, single submitter | clinical testing | The c.338dupT pathogenic mutation, located in coding exon 4 of the NF1 gene, results from a duplication of T at nucleotide position 338, causing a translational frameshift with a predicted alternate stop codon (p.L113Ffs*14). This alteration is expected to result in loss of function by premature protein truncation or nonsense-mediated mRNA decay. As such, this alteration is interpreted as a disease-causing mutation. |
Genome- |
RCV000793049 | SCV002561571 | pathogenic | Neurofibromatosis, type 1 | 2022-03-15 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV004549869 | SCV004800276 | pathogenic | NF1-related disorder | 2024-02-02 | no assertion criteria provided | clinical testing | The NF1 c.338dupT variant is predicted to result in a frameshift and premature protein termination (p.Leu113Phefs*14). To our knowledge, this variant has not been reported in the literature or in a large population database, indicating this variant is rare. Frameshift variants in NF1 are expected to be pathogenic. This variant is interpreted as pathogenic. |